The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment

BACKGROUND AND OBJECTIVE: Since being named and reclassified by WHO in 1996, solid-pseudopapillary tumor (SPT) of pancreas has been recognized as a special entitative disease that is different from pancreatic cancer and should be recognized and treated more accurately in the surgical process. The clinic characteristics and surgical strategy on 25 cases of SPT of pancreas from the authors' center are discussed. METHODS: The clinical pathology and the surgical methods of 25 SPTs were retrospectively studied. The analyses were performed by the statistical software package SAS6.12. RESULTS: No tumor recurrences were found in all patients. There was significant difference between operative types in radical resection and the tumor position of the pancreas (P = 0.0011). The judgment on the tumor's boundary could directly affect the adoptable operative types (P = 0.0099). CONCLUSIONS: As a uniquely entitative disease, SPT is a kind of uncommon neoplasm with low-grade malignancy with a strong rate of occurrence in women. Surgical resection is most favorable in the treatment of SPT, which has excellent prognosis. The course of SPT, the possible malignant cells by the frozen section biopsy, and the tumor's boundary are important for operators to decide an operative scheme. SPT that has infiltrated contiguous vessels, organs, even with local liver metastasis should not be regarded as operative contraindication. The choice of the local tumor resection, the part of pancreas resection or radical resection depends on the judgment of the tumor's boundary, whereas operative types in radical resection depend on the tumor position of the pancreas.     

Mixed granulosa-Sertoli sex cord-stromal tumor of the testis: clinical,ultrasonic and histopathological features: a case report

Testicular sex cord-stromal tumors are less common in men, while mixed sex cord-stromal tumors (MSCSTs) are rarer. Recently, we found a MSCST in an adult male testis [adult granulosa cell tumor (AGCT) with Sertoli cell tumor]. He was admitted to the hospital based on “left testicular bloating and dull pain for 20 years and aggravating for 10 days”. Routine examination of color Doppler ultrasound showed a size of approximately 1.09 cm × 0.79 cm in the left testis with a low echo area, clear outline, and color flow in it. The patient underwent a radical left orchiectomy to remove the tumor. Pathological results showed that the tumor was diagnosed as testicular MSCST (AGCT with Sertoli cell tumor). He was in good health after the operation and showed no signs of recurrence or metastasis after 6 months of follow-up. We summarized the clinical, ultrasonic, and histopathological characteristics of this case. And immunohistochemical staining was very important in the pathological diagnosis of testicular MSCSTs, which can distinguish different tumor types. MSCSTs were usually mixed Sertoli-Leydig cell tumors, while this case is a MSCST of AGCT with Sertoli cell tumor, which is unique from other cases. Moreover, in this case, the doctors could not clearly diagnose the tumor through pre-operative physical, ultrasonic and laboratory examinations until the postoperative pathological examination. This further reflected the importance of pathological examination in the diagnosis of such tumors.     

Functional paraganglioma with tumor thrombus in the inferior vena cava,first case report

Pheochromocytoma (PHEO) is a rare neuroendocrine that tumor originated from the adrenal medulla that secrets catecholamines. Tumors from extra-adrenal chromaffin tissues are called extra-adrenal PHEO or paraganglioma (PGL). To our knowledge, adrenal PHEO and subclinical PGL with inferior vena cava (IVC) invasion had been sporadically reported, while functional PGL with IVC tumor thrombus has not been publicly reported yet. Perioperative management of those diseases is less well established because of their multidisciplinary nature and rarity. We herein present a case of primary malignant PGL with IVC invasion. A 16-year-old female patient with a history of severe paroxysmal hypertension was admitted to Peking Union Medical College Hospital on suspicion of retroperitoneal mass. In-house diagnostic work-up revealed a malignant PGL with IVC invasion, inferior mesenteric artery encasement and, aorta engagement. Multi-disciplinary discussions were held and careful preoperative preparation plans were made. After everything was ready, the functional PGL and tumor thrombus were completely resected, then a reconstruction of IVC was performed. The patient was discharged on postoperative day 14 and all her clinical symptoms disappeared afterward. No evidence of tumor residual or metastasis was found in the subsequent six months of follow-up. Gene tests were made for her and her family. Albeit its rarity, functional PGL with IVC invasion is not unresectable, a multi-disciplinary task force should be established to settle down every detail. We recommended 3-dimensional imaging reconstruction for gaining a better anatomic understanding. Literature reviews showed that complete resection is the premise of a good prognosis. In particular cases, complementary or alternative therapy like chemotherapy and ¹³¹I-metaiodobenzylguanidine might help, family hereditary genetic tests are advised as well.     

Gastrointestinal stromal tumor of the posterior mediastinum

Gastrointestinal stromal tumor (GIST) is a rare, but potentially aggressive tumor. We present an asymptomatic 64-year-old man with an incidental 9-cm GIST that arose in the posterior mediastinum. Wide surgical excision was performed with rotation of an intercostal muscle flap to buttress a surgically created esophageal wall defect. The patient is now free of disease 26 months postoperative. This tumor is defined by the carcinogenic over-expression of KIT-protein, a tyrosine kinase receptor. Accurate diagnosis of gastrointestinal stromal tumor is imperative, as specific medical therapy is now available for potential control of recurrent or metastatic disease.     

胰血管活性肠肽瘤1例报告并国内文献复习

目的 探讨胰血管活性肠肽瘤（VIPoma）诊断和治疗方法。方法 报告中国医科大学附属第一医院2010年2月收治1例VIPoma的临床资料,并检索国内1993年1月至2009年12月间文献报道的24例。对25例VIPoma临床表现、实验室检查、影像学检查、手术方式及随访资料进行分析。结果 有记载的24例中22例有周期性发作的水样泻;22例检测血清钾,21例有低钾血症。13例血浆血管活性肠肽（vasoactive intestinal peptide,VIP）测定均高于正常。行B超检查15例中13例发现病变,10例病变于胰腺内。行CT检查22例中18例胰腺内发现病变,其中7例发现肝脏转移性病灶。25例中24例行手术治疗,行胰体尾切除术13例中11例术前有水样泻,术后7例水样泻消失,1例好转,另3例无记载。行肿瘤摘除术3例中1例术后水样泻消失,1例明显好转。1例胰头部VIPoma并发肝转移,行扩大的胰十二指肠切除术后水样泻消失。行胰体尾切除术13例,获得远期随访8例,随访3个月至3.5年,1例术后3个月大出血死亡,1例术后3.5年复发、肝转移,另6例无复发。结论 VIPoma诊断依赖于典型的临床症状和血浆VIP测定;B超及CT是胰腺VIPoma定位诊断的可靠方法。手术切除为胰腺VIPoma有效治疗手段,即使是姑息性切除亦可改善病人生活质量,延长存活时间。     

Solitary fibrous tumor of the inguinal region

Solitary fibrous tumors (SFT) have recently been established to be of mesenchymal origin. Although there are numerous reports of SFTs arising from the pleura, reports of the tumor arising from extrapleural sites are comparatively rare [Fletcher CDM, Unni K, Mertens F (eds) World Health Organization classification of tumors, pathology & genetics, tumors of soft tissue and bone. IARC Press, Lyon, pp 86–901, 2002]. We report a case of SFT arising in the inguinal region.     

Primary primitive neuroectodermal tumor of the kidney: A case report

There are nearly 50 PNET cases in the literature with primary renal localization. The mean age for renal PNET is 28 but it can be seen in a wide range between 4 and 69 years. In this atypical localization PNET may be mistaken with a variety of small round cell tumors, particularly with blastema predominant Wilms’ tumor and due to its distinctive prognostic and therapeutic features differential diagnosis is very important. Immunohistochemical studies are useful in discriminating PNET from other renal small round cell tumors. Renal PNETs tend to metastasize at early stages and they have a high rate of recurrence. Our case is a 45-year-old male presented with left flank pain and hematuria. In computerized tomography a mass in left kidney was observed and nephrectomy was performed. Histopathologic examination revealed a small round cell tumor with rosettes and pseudorosettes. Immunohistochemically the lesion was diffuse strong positive with CD99 and NSE, negative with LCA, pancytokeratin, vimentin, desmin, smooth muscle actin, chromogranine A and S-100. At the time of diagnosis the pathologic stage of the tumor was T1aN0Mx. The patient did not take any further therapy and in 1-year follow-up no local recurrence or distant metastasize occurred.     

Antitumor effects of recombinant human tumor necrosis factor against human tumor xenografts transplanted into nude mice

Antitumor activities of recombinant human tumor necrosis factor (rH-TNF) against human tumor xenografts in nude mice were studied. Thirteen human tumor xenografts serially transplanted into nude mice were used for experiments; five gastric, two breast, two gallbladder, one colon and one esophageal carcinoma, one liposarcoma and one squamous carcinoma of the neck. They were inoculated into the subcutaneous tissue of BALB/c nu/nu nude mice and the treatment was started when the estimated tumor weight reached 100–300 mg. rH-TNF was administered intratumorally at schedule of qd×5 or q3d×5. rH-TNF showed a marked antitumor activity against various human tumors. The hemorrhagic necrosis was observed in all types of the human tumor xenografts (100 per cent), and the complete regression of the tumor was noted in 4 of 11 tumors (36.4 per cent). On the contray, intraperitoneal rH-TNF exhibited little antitumor effect. The additive effect in the combination of TNF and Mitomycin C was observed against two Mitomycin C resistant gastric tumors.     

Residual tumor size and IGCCCG risk classification predict additional vascular procedures in patients with germ cell tumors and residual tumor resection: a multicenter analysis of the German Testicular Cancer Study Group

Background

Residual tumor resection (RTR) after chemotherapy in patients with advanced germ cell tumors (GCT) is an important part of the multimodal treatment. To provide a complete resection of residual tumor, additional surgical procedures are sometimes necessary. In particular, additional vascular interventions are high-risk procedures that require multidisciplinary planning and adequate resources to optimize outcome.

Objectives

The aim was to identify parameters that predict additional vascular procedures during RTR in GCT patients.

Design, setting, and participants

A retrospective analysis was performed in 402 GCT patients who underwent 414 RTRs in 9 German Testicular Cancer Study Group (GTCSG) centers. Overall, 339 of 414 RTRs were evaluable with complete perioperative data sets.

Measurements

The RTR database was queried for additional vascular procedures (inferior vena cava [IVC] interventions, aortic prosthesis) and correlated to International Germ Cell Cancer Collaborative Group (IGCCCG) classification and residual tumor volume.

Results and limitations

In 40 RTRs, major vascular procedures (23 IVC resections with or without prosthesis, 11 partial IVC resections, and 6 aortic prostheses) were performed. In univariate analysis, the necessity of IVC intervention was significantly correlated with IGCCCG (14.1% intermediate/poor vs 4.8% good; p = 0.0047) and residual tumor size (3.7% size <5 cm vs 17.9% size ≥5 cm; p < 0.0001). In multivariate analysis, IVC intervention was significantly associated with residual tumor size ≥5 cm (odds ratio [OR]: 4.61; p = 0.0007). In a predictive model combining residual tumor size and IGCCCG classification, every fifth patient (20.4%) with a residual tumor size ≥5 cm and intermediate or poor prognosis needed an IVC intervention during RTR. The need for an aortic prosthesis showed no correlation to either IGCCCG (p = 0.1811) or tumor size (p = 0.0651).

Conclusions

The necessity for IVC intervention during RTR is correlated to residual tumor size and initial IGCCCG classification. Patients with high-volume residual tumors and intermediate or poor risk features must initially be identified as high-risk patients for vascular procedures and therefore should be referred to specialized surgical centers with the ad hoc possibility of vascular interventions.     

Effect of myeloid ecotropic viral integration site (MEIS) family genes on tumor microenvironment remodeling and its potential therapeutic effect

BackgroundThe myeloid ecotropic viral integration site (MEIS) family of genes is related to the occurrence, development, and outcome of many cancers. However, its role in the immune and tumor microenvironment (TME) is unclear. This study explored the relationship between the expression of MEIS genes and patient survival, immune subtypes, TME, tumor stem cell correlation, and drug sensitivity in cancer.MethodsWe used The Cancer Genome Atlas pan-cancer data to analyze the expression of the MEIS family genes. Kaplan-Meier analysis and univariate Cox proportional hazard regression model were used to detect the relationship between gene expression and overall survival. Analysis of variance was used to explore the relationship between the MEIS family and the immune components in the tumor, and the ESTIMATE algorithm was used to calculate the proportion and level of tumor-infiltrating immune cells. Spearman and Pearson’s correlation tests were carried out to detect the relationship between MEIS and the characteristics of tumor stem cells and drug sensitivity.ResultsThe MEIS family of genes shows different expression profiles in different cancers, with substantial inter- and intra-cancer heterogeneity. Among them, MEIS3 was upregulated in most cancers, whereas MEIS2 was downregulated. The change in MEIS gene expression was usually related to overall survival, but whether a member of the MEIS family was a risk factor or a protective factor was cancer-dependent. Immune component analysis suggested that the role of MEIS genes in promoting or inhibiting cancer may be related to different degrees of immune silencing. Further, there were varying degrees of correlation between MEIS gene expression and cancer cell stemness characteristics. It was also found that MEIS genes, especially MEIS1 and MEIS2, may be related to chemotherapy resistance.ConclusionsWe explored the expression, prognostic relationship, molecular characteristics, and effects on immunity and TME of the MEIS gene family in cancer. Our results suggest that MEIS members should be studied as independent entities in different types of cancer. The MEIS gene family may be a potential target for cancer therapy, but further experiments are needed to confirm this.     

组织因子途径抑制物-2影响人前列腺癌细胞侵袭能力的实验研究

目的:探讨人组织因子途径抑制物2(TFPI-2)对基因转染入人前列腺癌细胞株PC3M体外和体内侵袭转移能力的影响.方法:通过Boyden小室体外侵袭转移模型,评价转染成功的PC3M-TFPI-2细胞和未转染的PC3M迁徙和侵袭能力的强弱;并且分别进行裸鼠接种实验,观察两组间其形成的局部新生物相对体积、组织浸润程度,以及有无远处转移的差异.结果:转染成功的PC3M-TFPI-2细胞中,证实有TFPI-2mRNA和相应蛋白质的表达,转染成功的PC3M-TFPI-2细胞较未转染的PC3M体外侵袭能力明显下降,接种PC3M-TFPI-2组细胞的裸鼠较接种未转染的PC3M的裸鼠形成的新生物肌层浸润明显减少.结论:人TFPI-2基因真核表达载体成功转染人PC3M并可以在PC3M中得到表达,TFPI-2的表达可以抑制PC3M的体外和体内的侵袭能力.     

复发性胃肠间质瘤再次手术

外科手术目前仍是胃肠间质瘤（GIST）病人获得根治的最佳手段,但术后复发转移是GIST病人治疗失败及死亡的主要原因。对于复发性GIST,外科治疗并不是最佳选择,应联合酪氨酸激酶抑制剂甲磺酸伊马替尼,结合病人的不同情况,个体化制定综合治疗方案,以提高病人总生存期。     

基因检测指导胃肠间质瘤治疗的价值

胃肠间质瘤（gastrointestinal stromal tumors,GIST）的发生与kit和PDGFR?α基因突变相关,有其独特的临床和病理特征。免疫组化的染色有助于GIST的诊断,而基因突变点的检测对临床应用伊马替尼和舒尼替尼治疗具有重要的指导意义。     

I型胶原吡啶交联终肽(ICTP)在骨肿瘤诊断中的临床研究

目的 检测良性骨肿瘤、恶性骨肿瘤患者及正常对照组血清中的I型胶原吡啶交联终肽(ICTP)活性，以评价ICTP在良恶性骨肿瘤诊断和鉴别诊断中的价值。方法 选取2005~2010年本院骨肿瘤科收治的78例恶性骨肿瘤患者，设为A组，其中49例为原发性恶性骨肿瘤、10例为继发性恶性骨肿瘤、19例为骨转移性肿瘤患者,另选43例良性骨肿瘤患者为B组，同时，设52例同龄正常人为C组，均采用酶免疫测定(EIA)方法测定他们血清中的ICTP活性。结果 B组患者血清ICTP活性为(6.75±3.34) μg/L，C组血清ICTP活性为(4.68±2.91) μg/L,A组患者血清ICTP活性为(14.84±8.49)μg/L，其中，原发性恶性骨肿瘤患者、继发性恶性骨肿瘤、骨转移性肿瘤患者的血清ICTP活性分别为(17.47±10.86)μg/L、(8.02±6.19) μg/L、(8.14±5.45) μg/L. A组中三类患者的血清ICTP活性与B组，C组相比差异均有统计学差异(P<0.05)，B组患者与C组相比差异无统计学差异(P>0.05)。而A组中原发性恶性骨肿瘤与继发性恶性骨肿瘤、骨转移性肿瘤组之间的差异也有统计学意义 (P<0.05)。结论 血清ICTP是反映骨肿瘤骨代谢的一个灵敏而简便的检测指标,并对良恶性骨肿瘤的诊断及鉴别诊断具有重要的临床意义。     

Development and validation of the prognostic value of the immune-related genes in clear cell renal cell carcinoma

BackgroundClear cell renal cell carcinoma (ccRCC) is a highly heterogeneous tumor, resulting a challenge of developing target therapeutics. Not long ago, immune checkpoint blockade regimens combine with tyrosin kinase inhibitors have evolved frontline options in metastatic RCC, which implies arrival of the era of tumor immunotherapy. Studies have demonstrated immune-related genes (IRGs) could characterize tumor milieu and related to patient survival. Nevertheless, the clinical significance of classifier depending on IRGs in ccRCC has not been well established.MethodsThe R package limma, univariate and LASSO cox regression analysis were used to screen the prognostic related IRGs from TCGA database. Multivariate cox regression was utilized to establish a risk prediction model for candidate genes. Quantitative real-time PCR was used to confirm the expression of candidates in clinical samples from our institution. CIBERSORT algorithm and correlation analysis were applied to explore tumor-infiltrating immune cells signature between different risk groups. A clinical nomogram was also developed to predict OS by using the rms R package based on the risk prediction model and other independent risk factors. The ICGC data was used for external validation of either gene risk model or nomogram.ResultsWe identified 382 differentially expressed immune related genes. Four unique prognostic IRGs (CRABP2, LTB4R, PTGER1 and TEK) were finally affirmed to associate with tumor survival independently and utilized to establish the risk score model. All candidates’ expression was successfully laboratory confirmed by q-PCR. CIBERSORT analysis implied patients in unfavorable-risk group with high CD8 T cell, regulatory T cell and NK cell infiltration, as well as high expression of PD-1, CTLA4, TNFRSF9, TIGIT and LAG3. A nomogram combined IRGs risk score with age, gender, TNM stage, Fuhrman grade, necrosis was further generated to predict of 3- and 5-year OS, which exhibited superior discriminative power (AUCs were 0.811 and 0.795).ConclusionsOur study established and validated a survival prognostic model system based on 4 unique immune related genes in ccRCC, which expands knowledge in tumor immune status and provide a potent prediction tool in future.     

食管源性转移瘤(癌)的现状回顾与归纳

食管源性转移瘤(癌)是指食管原发性恶性肿瘤经血道转移到人体各部位的组织或器官。发生移位状态的转移瘤(癌)是临床治疗的难题和预后不良的主要原因,也是目前肿瘤研究中的重要领域和热点。该文收集了国内的相关资料,对其发病现状、发生机制、影响因素、早期诊断及治疗原则进行回顾与归纳。